Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hypoxemia, hypercarbia, and cor pulmonale ultimately occur in most patients with chronic lung disease. Although oxygen therapy may reduce or delay the development of pulmonary hypertension and myocardial failure in these patients, its use is thought to lead to CO2 narcosis and apnea. The effect of O2 administration during sleep has been examined in 12 patients (seven with cystic fibrosis, three with bronchopulmonary dysplasia, one with bronchiolitis obliterans, and one with severe hypersensitivity pneumonitis) using skin surface O2 (Roche) and CO2 (Radiometer) electrodes. Both electrodes were calibrated over wet gas and applied at 44 C. Ten patients had chronic hypercarbia (PaCO2 62 +/- 19 torr; range 46 to 103 torr) when awake. Humidified oxygen was administered by nasal cannula, Venturi mask, or head hood. Oxygen flow was increased every 20 minutes from 80 minutes or until the patient awoke. In eight of ten patients with hypercarbia and in the two normocarbic patients, skin surface carbon dioxide tension (PsCO2) increased by 10% or less as the skin surface oxygen tension (PsO2) was increased. In the remaining two patients with hypercarbia (both had cystic fibrosis) PsCO2 increased 18% and 24% as PsO2 was increased. These last two patients with depressed responsiveness to CO2 could not be separated from the other patients by clinical or laboratory criteria. It is concluded that the skin surface blood gas tensions are a simple and reproducible method for adjusting oxygen therapy in patients with chronic lung disease, and although the response to oxygen varies from patient to patient, most patients with chronic hypercarbia retain their central responsiveness to CO2 during sleep and for them O2 therapy is probably safe.
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PMID:Effect of oxygen administration during sleep on skin surface oxygen and carbon dioxide tensions in patients with chronic lung disease. 678 98

We report the case of a 9 month-old infant in whom acute heart failure was the presenting symptom of cystic fibrosis. Post-mortem examination showed lesions of myocardial fibrosis associated with typical lesions of cystic fibrosis in the pancreas. Hypoproteinemia and anemia were present and earlier could have suggested the correct diagnosis before the episode of acute heart failure. The analogy of the histologic myocardial changes in cystic fibrosis and in congenital lipomatosis of the pancreas suggests a common pathogeny related to the deficiency of the exocrine secretion of the pancreas.
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PMID:[Myocardial fibrosis in cystic fibrosis. A new case report (author's transl)]. 706 17

The ability of digoxin to increase exercise capacity and stroke volume (SV) during exercise was evaluated in ten patients with cystic fibrosis (CF) ages 12 to 20 years with moderate to severe degrees of airway obstruction but no history of heart failure. A double-blind crossover trial of digoxin versus placebo was carried out. An evaluation of exercise performance was undertaken upon entry into the study, and after each of the one-week periods in which digoxin 0.25 mg/day or placebo was taken. Exercise testing consisted of a progressive exercise test on a cycle ergometer to measure maximum work capacity (Wmax) and a steady state test at 2/3 of the baseline Wmax. During the steady state test, the oxygen consumption and carbon dioxide production were measured and cardiac output (Q) was calculated by the indirect Fick (CO2) method. From Q and heart rate (HR), SV was derived. After digoxin, Wmax was unchanged. On steady state exercise HR was unchanged, but there was a slight but significant fall in Q due to a fall in SV. The decrease in SV was associated with exercising hypoxemia. We conclude that digoxin did not increase exercise capacity or improve exercising cardiac function in patients with moderate to severe airway obstruction due to CF.
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PMID:The effect of digoxin on exercise capacity and exercising cardiac function in cystic fibrosis. 712 21

Progress over the last 40 years has greatly reduced morbidity and mortality in the constantly changing field of thoracic surgery. The first part of this review focuses on current indications and limitations in lung surgery. Technical procedures for pneumonectomy, lobectomy, bronchial resection and conservative surgery are well established. Although major respiratory or cardiac failure still limit indications bronchogenic cancer extension is no longer a contraindication. Exeresis after 70 years of age is not an exception. Surgery for non-small cell lung cancer has given promising results with a 5-year survival rate of 60-80% for patients in stage I and II. For stage III, two recent comparative studies have demonstrated the effectiveness of preoperative adjuvant chemotherapy which should logically be proposed with or without radiotherapy in patients with resectable tumours. Surgical removal of lung metastases and mesotheliomas has also made considerable progress. Unfortunately, except for therapeutic trials, exeresis of small cell lung cancer does not provide any beneficial effect and cannot be proposed. Indications for surgery in patients with chronic obstructive pulmonary disease however has been quite successful and now goes beyond classical exeresis of large compressive bullae. In many situations patients with diffuse emphysema can benefit from surgical reduction in lung volume before proposing transplantation. Lung transplantation is indicated for pulmonary fibrosis, pulmonary vascular disease and obstructive lung pulmonary disease with an overall survival rate of 50% at 5 years and 43% at 6 years. The rate of successful bilateral lung transplantation for cystic fibrosis remains to be determined.
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PMID:[New techniques in thoracic surgery. I]. 756 9

Left ventricular failure is not considered an important feature in cystic fibrosis (CF), but abnormalities of left ventricular function have been reported. Except for a few cases of heart failure in neonates with CF, there is no evidence of a primary disorder of the myocardium in patients with CF. Since left ventricular perfusion disturbances can cause left ventricular dysfunction, we decided to investigate left ventricular perfusion during exercise using sestamibi-Tc-99m-labeled macroaggregates. Eighteen CF patients with varying degrees of disease severity participated in the study. They underwent a thorough clinical evaluation, lung perfusion scan, pulmonary function testing, echocardiography, transcutaneous measurement of oxygen saturation at rest and during exercise, and an exercise test with injection of sestamibi-Tc-99m-labeled macroaggregates at peak exercise. Six patients (33%) showed abnormalities of the myocardial distribution of sestamibi-Tc-99m-labeled macroaggregates during exercise. Scanning abnormalities correlated with the clinical score, mean maximum expiratory flow at 50% of vital capacity (MEF50), and arterial oxygen desaturation during exercise. We conclude that deficits in left ventricular uptake of sestamibi-Tc-99m-labeled macroaggregates during exercise seem common in patients with severe CF lung disease. The cause of these deficits is not fully understood, but the occurrence seems to be associated with a poor prognosis.
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PMID:Left ventricular perfusion deficit in patients with cystic fibrosis. 951 92

Acute infections of the lower respiratory tract first require a weighing up of risks, which is of importance in particular for the decision for or against antibiotic therapy. Severe or longlasting exacerbations of a chronic obstructive bronchitis, severe and rapidly progressive bronchial asthma or infection associated with bronchiectasis in an underlying antibody deficiency syndrome, primary ciliary dyskinesia and mucoviscidosis. In the case of systemic immunodeficiencies such as the antibody deficiency syndrome, HIV infection or immunosuppressive therapy, the indication for antibiotic treatment is more liberally established. In combination with respiratory tract infections, serious underlying disease such as left heart insufficiency or diseases of the lungs, may become life-threatening. Of decisive importance for the outcome in such cases are, besides the use of antibiotics, such as treatment of the cardiac insufficiency or long-term oxygen therapy. Timely vaccination can prevent severe illness.
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PMID:[Acute infection of the lower respiratory tract: how long to observe?]. 1113 86

Cystic fibrosis is the most common life-limiting recessive genetic disorder in Caucasian. It is caused by mutations of CFTR gene (cystic fibrosis transmembrane conductance regulator); at present over 500 mutations are known. Cystic fibrosis as a cause of respiratory distress in the neonate is quite rare. In neonatal period the most important clinical manifestations are meconium ileum and much rarely cholestatic jaundice. We present two cases of cystic fibrosis in newborns. In the first one, we point out the strict association between meconium ileum and cystic fibrosis. The patient underwent a surgical treatment for meconium ileum and the diagnosis was rapidly confirmed by genetic analysis and sweat test. The second one had intestinal obstruction from birth caused by meconium ileum associated with ileal atresia; besides, he developed cholestatic jaundice, severe and rapidly progressive respiratory disease. He died at 102 degrees day of age for cardiac failure. The diagnosis of cystic fibrosis, supported by typical clinical features and high level of serum trypsin, unfortunately wasn't confirmed by genetic analysis (lambda F508/neg), in addition, the sweat test wasn't reliable because an inadequate quantity of sweat was collected.
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PMID:[Neonatal cystic fibrosis: report of 2 cases]. 1142 48

Multifocal myocardial necrosis (MMN) is an unusual cardiomyopathy of childhood, characterized by multiple patchy areas of myocardial fiber necrosis/fibrosis involving mainly the middle part of the left ventricle, but also, to a lesser extent, the right ventricle and the atria. These necrotic lesions are isolated and are not accompanied by an inflammatory reaction or vascular alterations. They are responsible for acute cardiac failure. MMN lesions are observed in various pathologic conditions including cystic fibrosis of the pancreas, pancreatic lipomatous hypoplasia/atrophy, malnutrition due to extensive intestinal resection with subsequent total parenteral feeding, and in Keshan disease. MMN is the main and the most characteristic feature of Keshan disease, an endemic and idiopathic condition affecting Chinese rural children. The causes and mechanisms of MMN presently are unknown. However, the presence of similar cardiac lesions in such different pathological conditions suggests the role of a selective deficiency of a hypothetical extrinsic factor (selenium, molybdenum iodide, other), probably crucial for the metabolism of the myocardial fiber.
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PMID:Multifocal myocardial necrosis: a distinctive cardiac lesion in cystic fibrosis, lipomatous pancreatic atrophy, and Keshan disease. 1255

Bronchoscopy is a highly versatile technique in the context of intensive care and has many potentially valuable indications. Safety is of paramount importance and the risks in critically unstable patients are correspondingly greater than in more stable children. The main contraindication to bronchoscopy is if it will provide no useful information. The procedure is obviously more risky in children with severe hypoxia, uncontrolled bleeding diathesis, cardiac failure or severe pulmonary hypertension. Monitoring should include at least oxygen saturation, blood pressure (ideally by continuous, invasive monitoring) and preferably capnography. Indications for bronchoscopy in paediatric intensive care include endobronchial toilet, sometimes instilling recombinant human DNAase even in children who do not have cystic fibrosis; checking tube patency and position; assisting in a difficult intubation or tube change; achieving the selective intubation of a main bronchus; the diagnosis and management of ventilator-associated pneumonia or the ventilated, immunocompromised host; the assessment of lobar collapse or focal hyperinflation; airway stent assessment; assessment of stridor on extubation and the diagnosis of any associated disease. New iatrogenic complications are also likely to be discovered. The procedure is very safe if performed by experienced operators with back-up from doctors skilled in airway management and the monitoring of sick children.
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PMID:Bronchoscopy in paediatric intensive care. 1261 34

In comparison to cation (K+, Na+, and Ca2+) channels, much less is currently known about the functional role of anion (Cl-) channels in cardiovascular physiology and pathophysiology. Over the past 15 years, various types of Cl- currents have been recorded in cardiac cells from different species including humans. All cardiac Cl- channels described to date may be encoded by five different Cl- channel genes: the PKA- and PKC-activated cystic fibrosis tansmembrane conductance regulator (CFTR), the volume-regulated ClC-2 and ClC-3, and the Ca2+-activated CLCA or Bestrophin. Recent studies using multiple approaches to examine the functional role of Cl- channels in the context of health and disease have demonstrated that Cl- channels might contribute to: 1) arrhythmogenesis in myocardial injury; 2) cardiac ischemic preconditioning; and 3) the adaptive remodeling of the heart during myocardial hypertrophy and heart failure. Therefore, anion channels represent very attractive novel targets for therapeutic approaches to the treatment of heart diseases. Recent evidence suggests that Cl- channels, like cation channels, might function as a multiprotein complex or functional module. In the post-genome era, the emergence of functional proteomics has necessitated a new paradigm shift to the structural and functional assessment of integrated Cl- channel multiprotein complexes in the heart, which could provide new insight into our understanding of the underlying mechanisms responsible for heart disease and protection.
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PMID:Functional role of anion channels in cardiac diseases. 1571 21


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